Sickle Cell Anemia (Exam 3) Flashcards
Who is at high risk for sickle cell trait/disease?
blacks
What is the main cause of sickle cell RBCs?
genetic mutation
What is the genetic mutation of sickle cell RBC and on which allele?
single base (A to U) substitution on amino acid position 6 of beta allele | glutamic acid to valine
What is the lab findings of someone who has sickle cell?
normochromic, normocytic RBC | Howell-Jolly | target cells
What is the lab findings of someone who is or has gone through a sickle cell crisis?
poikilocytosis | anisocytosis | nucleated RBC in blood | sickle cell RBC
What is the main cause of a sickle cell crisis?
low pO2
What is a sickle cell crisis?
crystallization of Hb in RBC = RBC into sickle shape (irreversible) and in-vivo RBC lysis
What is the most common complaint of someone who has a sickle cell crisis? And why?
pain due to the sickle RBC blocking circulation = O2 is not getting into tissue = cells die and replaced with fiber
What is the cause of death in sickle cell anemia?
organ failure due to tissue necrosis because the organ function was compromised
How early can a child with sickle-cell anemia show symptoms? Why?
6 months after birth = when HbF turns into HbS
What are the 5 events that can induce sickle cell crisis?
smoking | high altitudes | infection | dehydration | medications
What are the 3 things that people with sickle cell anemia are susceptible to?
infection | infarction (blockage) | retardation growth
What are the 2 laboratory tests used to determine if someone has the HbS RBCs?
Hb electrophoresis | Metabisulfite test
What is the metabisulfite test?
metabisulfite binds to O2 = neutralizing O2 in a patient = induce sickle cell in patient
What are the 4 methods of treatment for Sickle Cell Anemia?
supportive | gene therapy | stem cell therapy | hydroxyuria
